Confirm all required screenings are completed and eligibility is confirmed prior to scheduling a patient’s infusion
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Call 1-833-436-0021 Monday through Friday, 8 AM-8 PM ET.
DOSING AND ADMINISTRATION FOR HEMGENIX
HEMGENIX is administered as a one-time-only intravenous infusion— delivered in 1-2 hours in an outpatient setting. No special pretreatment of patients is required.
HEMGENIX should only be prepared and administered by trained staff at an administration center
Interested in becoming an administration center? Contact your local CSL Behring representative, call HEMGENIX Connect℠ at 1-833-436-0021, Monday through Friday from 8AM–8PM ET or complete the form here
Each dosing kit is personalized to your patient’s weight
HEMGENIX is delivered as a single dose of 2 mL/kg body weight*
HEMGENIX does not contain preservatives. Use aseptic technique and proper engineering controls (eg, a biological safety cabinet or isolator) according to institutional policies during the preparation and administration of HEMGENIX
Questions along the way? Call HEMGENIX Connect℠ at 1-833-436-0021, Monday through Friday from 8 AM-8 PM ET.
Questions along the way? Call HEMGENIX Connect℠ at 1-833-436-0021, Monday through Friday from 8 AM-8 PM ET.
Preparing for Infusion
Confirm all required screenings are completed and eligibility is confirmed prior to scheduling a patient’s infusion
Complete and submit the prescription referral form for HEMGENIX or coordinate the referral to another administration center
Consult with patients to schedule their infusion day. Remind them they need to maintain a consistent weight from prescription to administration
HEMGENIX Connect℠ will work with patients to verify the administration center is within insurance coverage, as well as provide other support and services
Locate an administration center or contact HEMGENIX Connect at 1-833-436-0021.
If the administration center is not local for your patient, they may need to plan on spending 1-2 days there, so patients should be prepared for the time commitment
Review infusion day expectations with your patient, including potential infusion-related reactions
Once your patient’s infusion day is set, schedule and confirm their post-treatment monitoring appointments and the lab you’ll use, and reinforce the importance of post-monitoring progress with HEMGENIX
Supplies for preparation
Supplies for administration
HEMGENIX is provided as a customized kit to meet dosing requirements for each patient, with each kit containing 10 to 48 10-mL vials of HEMGENIX. The total number of vials in each kit corresponds to the dosing requirement for the individual patient depending on the patient’s body weight.
HEMGENIX should only be diluted just prior to administration. Keep the packaging sealed until the patient and full administration team are ready.
On the day of the infusion:
Before you begin, verify that:
Prior to dilution, spike the infusion bag(s) of 0.9% normal saline solution with applicable connector.
Connect a Luer-lock syringe at the mixing adapter site of the applicable connector.
Withdraw the volume equal to the calculated HEMGENIX dose (in mL) from the 500 mL infusion bag(s) of 0.9% normal saline solution. The volume to be withdrawn and number of infusion bag(s) needed will vary based on the patient body weight:
| Patient body weight | Number of 500 mL 0.9% normal saline infusion bag(s) required | Volume of saline solution to withdraw |
|---|---|---|
| Less than 120kg body weight | One | Equal to the total HEMGENIX dose (in mL) from one bag |
| Equal to or more than 120 kg body weight | Two | Equal to the total HEMGENIX dose (in mL). Remove half of the dose equivalent volume from each of the two bags |
HEMGENIX injection to the 0.9% normal saline infusion bags
Prior to dilution, inspect each of the HEMGENIX single-dose vials.
Gently swirl the vials 3 times (about 10 seconds) to homogenize the HEMGENIX suspension.
Remove the plastic flip-off cap from the vials and disinfect the rubber stopper with a sterilizing agent (for example sterile 70% isopropyl alcohol).
Withdraw HEMGENIX from each vial using a 20 G needle/vial adapter and syringe.
Slowly add the required HEMGENIX dose from the syringe(s) directly to the 0.9% normal saline solution in the infusion bag(s) (from step #3) to bring the total volume in each infusion bag back to 500 mL.
Repeat steps 7 and 8 with additional needles/vial adaptors and syringes to inject the total calculated HEMGENIX volume to the infusion bag(s) required for the patient dose.
Gently invert the infusion bag(s) at least 3 times (about 10 seconds) to mix the solution and ensure even distribution of the diluted product.
Label the infusion bag(s)
Connect the infusion bag(s) to an infusion tube pre-filled with sterile 0.9% normal saline solution to reduce the risk of spillage and/or aerosol formation.
Transport the diluted HEMGENIX infusion bag(s) in the transport container/bag protected from light to the administration site, avoiding any shaking or excessive agitation.
Administration
Visually inspect
Visually inspect diluted HEMGENIX solution prior to administration. The diluted HEMGENIX should be clear and colorless.
Use an integrated filter
Use an integrated (in-line) 0.2-mcm filter made out of polyether sulfone (PES).
Prepare the line
Connect the prefilled IV infusion line/drip chamber to the main intravenous line, which has been primed with sterile 0.9% normal saline solution prior to use.
Infuse at a constant rate
Infuse the diluted HEMGENIX solution at a constant infusion rate of 500 mL/hour (8 mL/min).
In the event of an infusion reaction* during administration:
The rate of infusion may be reduced or stopped to manage the infusion reaction. If the infusion is stopped, restart at a slower rate when the infusion reaction is resolved.
If the infusion rate needs to be reduced, or stopped and restarted, HEMGENIX should be infused within 24 hours after dose preparation.
* Symptoms may include chest tightness, headaches, abdominal pain, lightheadedness, flu-like symptoms, shivering, flushing, rash, and hypertension.
Ensure all HEMGENIX is delivered
After the entire content of the bag(s) is infused, flush the infusion line and the infusion tubing at the same infusion rate (8 mL/min) with 0.9% normal saline solution to ensure all HEMGENIX is delivered.
Closely monitor patients for infusion reactions throughout the infusion period and for at least 3 hours after the end of the infusion.
Post-monitoring and tracking outcomes
After administration of HEMGENIX, you and your patients will have frequent follow-ups to monitor progress.
Liver enzymes: Check for potential elevations that may require intervention
Factor IX activity: Monitor factor IX activity. Use of exogenous factor IX concentrates before and after administration may impede assessment of endogenous, HEMGENIX-derived factor IX activity. It may take several weeks after treatment with HEMGENIX for endogenous factor IX levels to rise. Until then, infusions of factor IX replacement product may be needed
Factor IX inhibitors: Post-dose inhibitor testing should be performed if bleeding is not controlled or if plasma factor IX activity levels decrease
Long-term liver health: Perform regular alpha-fetoprotein (AFP) level testing and abdominal ultrasound (eg, annually) in patients with preexisting risk factors for hepatocellular carcinoma
You and your patient can create a follow-up plan that works best for you both, making it convenient with routine visits, local lab draws, and telemedicine appointments.
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Patient Selection
Perform baseline testing to select patients, including titer testing for Factor IX (FIX) inhibitor presence. Do not administer HEMGENIX®, etranacogene dezaparvovec-drlb, to patients with FIX inhibitors or a history of FIX inhibitors. Perform liver health assessments, consulting with a hepatologist if needed. Also perform laboratory tests to evaluate hepatitis B and C, and postpone treatment if patient has active infection, as this may reduce the efficacy of HEMGENIX and/or increase the risk of adverse reactions.
Warnings and Precautions
Hypersensitivity and Infusion Reactions
Infusion reactions, including hypersensitivity reactions and anaphylaxis, have occurred. Monitor during administration and for at least 3 hours after end of infusion. If symptoms occur, slow or interrupt administration. When symptoms have resolved, restart administration at a slower infusion rate.
Hepatotoxicity/Hepatocellular Carcinogenicity
Hepatotoxicity with elevated liver transaminase has occurred after HEMGENIX treatment. Monitor ALT levels once per week for 3 months and thereafter monthly up to 1 year after administration. Consider corticosteroid treatment should elevations occur and as clinically indicated.
The integration of liver-targeting AAV vector DNA into the genome may carry the theoretical risk of hepatocellular carcinoma development. For patients with preexisting risk factors for hepatocellular carcinogenicity, consider liver ultrasound and alpha-fetoprotein testing following administration, and monitor for hepatocellular carcinomas for five years following administration of HEMGENIX.
Immune-Mediated Neutralization of the AAV5 vector capsid
Preexisting neutralizing anti-AAV antibodies may impede transgene expression at desired therapeutic levels. Following treatment with HEMGENIX, all patients developed neutralizing anti-AAV5 antibodies to AAV5 vector capsid.
Monitoring Laboratory Tests
Monitor patients regularly for FIX activity (eg, weekly for 3 months), especially when exogenous FIX is administered, as it may take several weeks following HEMGENIX administration before hemostatic control becomes apparent. Hemostatic support may be needed for some patients. Monitor patients through appropriate clinical observations and laboratory tests for the development of inhibitors to FIX.
Adverse Reactions
The most common adverse reactions (incidence ≥5% in clinical trials) are elevated ALT, headache, blood creatine kinase elevations, flu-like symptoms, infusion-related reactions, fatigue, nausea, malaise, and elevated AST.
Indication
HEMGENIX is indicated for the treatment of adults with Hemophilia B (congenital Factor IX deficiency) who:
HEMGENIX is for single-use intravenous infusion only.
Please see full prescribing information for HEMGENIX.
To report SUSPECTED ADVERSE REACTIONS, contact the CSL Behring Pharmacovigilance Department at 1-866-915-6958 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
